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Science 18 July 1986:
Vol. 233. no. 4761, pp. 364 - 367
DOI: 10.1126/science.3014653

Articles

Science, Vol 233, Issue 4761, 364-367
Copyright © 1986 by American Association for the Advancement of Science


articles

Human prion protein cDNA: molecular cloning, chromosomal mapping, and biological implications

YC Liao, RV Lebo, GA Clawson, and EA Smuckler

A human complementary DNA whose protein product is considered to be the major component of scrapie-associated fibrils in Creutzfeldt-Jakob disease, kuru, and Gerstmann-Straussler syndrome has been identified and characterized. The extensive homology of this gene sequence to the hamster PrP 27- to 30-kilodalton prion protein complementary DNA clone, and its existence as a single copy in the human genome, leads to the conclusion that this is the human prion gene. This human prion gene has been mapped to human chromosome 20, negating a direct link between the prion protein and Down's syndrome or the amyloid of Alzheimer's disease.


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Identification of Novel Proteinase K-resistant C-terminal Fragments of PrP in Creutzfeldt-Jakob Disease.
W.-Q. Zou, S. Capellari, P. Parchi, M.-S. Sy, P. Gambetti, and S. G. Chen (2003)
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An Orthodox Heresy: Scientific Rhetoric and the Science of Prions.
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Lingering Doubts about Spongiform Encephalopathy and Creutzfeldt-Jakob Disease.
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Experimental Biology and Medicine 226, 640-652
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Effect of the E200K Mutation on Prion Protein Metabolism : Comparative Study of a Cell Model and Human Brain.
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Prion Protein Structure and Scrapie Replication: Theoretical, Spectroscopic, and Genetic Investigations.
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Truncated Forms of the Human Prion Protein in Normal Brain and in Prion Diseases.
S. G. Chen, D. B. Teplow, P. Parchi, J. K. Teller, P. Gambetti, and L. Autilio-Gambetti (1995)
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Molecular biology of prion diseases.
S. Prusiner (1991)
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Spontaneous neurodegeneration in transgenic mice with mutant prion protein.
K. Hsiao, M Scott, D Foster, D. Groth, S. DeArmond, and S. Prusiner (1990)
Science 250, 1587-1590
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